AB0720 Adult Onset Still’s Disease: Points to Consider
Authors: L. Martin Calderon, J. Pope
Journal: Annals of the Rheumatic Diseases (2022)

Background Adult-onset Still’s disease (AOSD) is a rare and complex autoinflammatory disease of unknown etiology. AOSD has a heterogeneous presentation which can lead to diagnostic uncertainty with prolonged time prior to treatment. NSAIDs, glucocorticoids, conventional DMARDs, and biologics are used in the management of AOSD with varying results. Furthermore, factors associated with de-escalation of therapies remain unclear. Objectives To review the literature and create a consensus on points to consider in the diagnosis, prognosis, and treatment of AOSD. Methods A scoping review of the AOSD literature over the last 15 years was performed. MEDLINE and EMBASE were searched and studies included if they provided information regarding the epidemiology, differential diagnosis, diagnostic criteria, complications, prognosis, and initial, chronic, and refractory treatment approaches in AOSD. Following narrative information synthesis, a meeting was held with experienced clinicians across Canada for the creation of a consensus on points to consider in AOSD. Results The annual incidence and prevalence of AOSD is observed to be between 0.16 to 0.62 per 100,000 and 3.9 to 6.9 per 100,000, respectively. AOSD most commonly affects young adults and women. Women are more likely to have severe complications from AOSD including macrophage activation syndrome, disseminated intravascular coagulation, or thrombotic thrombocytopenic purpura. The Yamaguchi criteria remains the most widely used diagnostic tool with a sensitivity of 96.2% and specificity of 92.1%. Common presentation manifestations include intermittent high fevers (&gt;39.0 degrees Celsius), arthralgias/arthritis, pharyngitis, lymphadenopathy, and a maculopapular rash. Other manifestations can variably involve the cardiovascular, respiratory, and GI systems. Common laboratory abnormalities include leukocytosis with neutrophilia, elevated ESR and CRP, elevated ferritin, and transaminitis. AOSD patients are most commonly ANA and Rf negative. Initial treatment includes NSAIDs, glucocorticoids, and conventional DMARDs. Disease refractory to initial therapy is managed through IL-1 and IL-6 inhibitors such as Anakinra or Tocilizumab. Elevated ESR, pericarditis, and non-response to corticosteroids are some of the factors associated with refractory and chronic disease requiring advanced therapies and long term follow-up. Conclusion AOSD is a multi-faceted autoinflammatory disease with a diverse presentation profile. Clinicians are recommended to consider AOSD, following exclusion of infections, malignancies, and autoimmune diseases, as a cause for fever of unknown etiology. Our review provides points to consider in the diagnosis and management of AOSD following expert consensus. References [1]Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine. 2014 Mar;93(2). [2]Sfriso P, Priori R, Valesini G, Rossi S, Montecucco CM, D’Ascanio A, et al. Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clinical rheumatology. 2016 Jul;35(7). Disclosure of Interests None declared